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PubMedDate not supplied

Cutaneous paraneoplastic syndromes.

Paraneoplastic syndromes are caused by hormones or other substances produced by cancer cells and may be the first sign of cancer. A wide range of paraneoplastic syndromes, including endocrine, neurologic, and cutaneous disorders, occurs in patients with cancer. More than 30 cutaneous paraneoplastic syndromes have been identified; this article reviews some of the more common syndromes--acanthosis nigricans, Paget's Disease, acquired ichthyosis, telangiectasia, hypertrichosis lanuginosa acquisita, erythroderma, Bazex's Syndrome, and necrotizing migratory erythema. When these syndromes are diagnosed during the course of a malignancy, professional caregivers may misinterpret them as indicative of metastatic disease or other disorders and patients may be misdiagnosed and not receive optimal treatment. Paraneoplastic syndromes also compromise quality of life by often causing skin impairment and discomfort. Therefore, nurses must be aware of the signs and symptoms of these cutaneous disorders and know how to care for patients with paraneoplastic syndromes.

Humans↗
PubMed1992

Nervous system dysfunction in children with paraneoplastic syndromes.

Paraneoplastic syndromes are complexes of symptoms and signs that occur in association with cancer and that are unexplained by the known anatomic and physiologic characteristics of the tumor. Many of these syndromes are neurologic in nature or have consequences for the central or peripheral nervous system. These syndromes have been well characterized in adults. With the exception of opsoclonus-myoclonus, little has been written about the occurrence of such syndromes in children. This review looks at published reports of paraneoplastic syndromes in children and concludes that paraneoplastic syndromes in childhood differ from those seen in adulthood because of differences in both the host and the kinds of neoplasms most prevalent in each age group. Paraneoplastic syndromes may be underreported in childhood because of the difficulty in eliciting specific neurologic complaints from children and because a thorough neurologic examination is often not undertaken as a matter of routine.

Child↗
PubMed2001

[Lung cancer and paraneoplastic syndromes].

Paraneoplastic syndromes (PNS) are a relatively common manifestation of cancer, and in some cases they may be the first symptom. Lung cancer has the highest incidence of paraneoplastic syndrome. This fact is important considering a non explained endocrinological and neurological syndrome, it may facilitate a prompt diagnosis, and in some cases an adequate treatment. PNS evolution seems to be parallel to the subjacent cancer. PNS management requires specific measures, because in some cases, it may compromise the patient life. Neurological and endocrinological PNS associated to lung cancer are revised, and diagnosis and treatment of them are updated.

Adrenocorticotropic Hormone↗
PubMed1997

Neurological paraneoplastic syndromes.

Paraneoplastic neurological syndromes are uncommon, however, their diagnosis is of major practical importance. The identification of antibodies in the serum or cerebrospinal fluid in central nervous system paraneoplastic syndromes confirms the clinical diagnosis of a paraneoplastic syndrome and allows early identification of an underlying tumour at a stage when it is localised and more amenable to treatment. The failure to identify antibodies in patients with characteristic presentations of underlying neurological paraneoplastic syndromes does not exclude an underlying cancer. Necrotising myelopathy, dermatomyositis, and chronic inflammatory demyelinating polyneuropathy all occur more frequently than expected in patients with cancer but autoantibodies have not yet been identified. Although significant advances have been made in diagnosis, further research is needed in the detection of autoantibodies and the elucidation of their role in the aetiology of neurological disease.

Autoantibodies↗
PubMed1998

Immunoadsorption therapy for paraneoplastic syndromes.

Paraneoplastic neurologic syndromes associated with systemic cancer are being increasingly recognized. Although these syndromes are thought to be immunologically mediated treatment with steroids, immunoglobulin and plasmapharesis has been disappointing. Based on our preliminary experience with the treatment of 6 cases of paraneoplastic neurologic syndromes with protein A immunoadsorption, an institutional, open-arm treatment protocol was established. Since our original report we have treated an additional 7 patients with this method. The 13 cases were accrued over a 2 year period and included 10 women and 3 men with an average age of 63. The paraneoplastic syndromes included 6 cases of cerebellar degeneration, 3 cases of opsoclonus/myoclonus, 3 cases of encephalomyelitis and 1 case of Lambert Eaton myasthenic syndrome. Primary cancers included 4 cases of small cell lung cancer, 2 cases of breast cancer, 2 cases of lymphoma and 1 each of acinic cell cancer, cholangiocarcinoma, Merkel cell cancer, pancreatic adenocarcinoma and rectal cancer. Anti-neuronal antibody status, cerebrospinal fluid and neuroimaging studies as well as cancer staging and treatment protocols were reviewed. Neurologic syndromes were clinically separated into component symptoms and signs for assessment of treatment effect. The treatment goal was a total of 6 sessions of protein A immunoadsorption given twice weekly. Twelve of 13 patients completed therapy and one patient developed cutaneous vasculitis during the second session with termination of treatment. Of the remaining patients 3/12 had a complete response of the primary clinical symptom/sign while 6/12 had a partial response for a total response rate of 9/12 (75%). Toxicity was limited to cutaneous vasculitis in 1 patient and an episode of hemisensory changes in another patient. Current treatment of paraneoplastic neurologic syndromes remains unsatisfactory. Despite the small number of patients in this report, protein A immunoadsorption is a promising therapy which deserves further study in a larger population of patients with paraneoplastic syndromes.

Adsorption↗
PubMed1988

Bilateral Baker's cyst as the presenting symptom of paraneoplastic syndrome.

Paraneoplastic syndrome is defined as a systemic malignancy producing prostaglandins or other substances that lead to various manifestations, syndromes or diseases. In the following report we present a case of a young patient complaining of bilateral Baker's cysts who ultimately was diagnosed as suffering from gastric lymphoma. Following subtotal gastrectomy the Baker's cysts disappeared with no specific treatment.

Adult↗
PubMed1997

Paraneoplastic syndromes.

Paraneoplastic syndromes (i.e. organ/tissue disorders associated with cancer) affecting the nervous system are thought to be the result of an autoimmune response triggered by specific cancer antigens. Several of these antigens have recently been identified and include the Hu, Yo and Ri proteins, with the Hu antigens being the best studied. Immunization of animals with HuD has been shown to retard the growth of HuD-positive neuroblastomas. In addition, the presence of anti-HuD antibody in humans with small-cell lung cancer predicts the slow growth of the tumor. The associated neurological disorders, however, limit the use of these and other antigens with similar characteristics in cancer vaccines.

Humans↗
PubMed1996

[Optimal fixation for the detection of anti-neuronal antibody by immunohistochemistry in the paraneoplastic syndrome].

Paraneoplastic syndrome(PS) associated anti-neuronal autoantibodies are characterized by their antigen molecular weight determined by Western blot and immunostaining pattern recognized through immunohistochemistry. We investigated immunohistochemical fixatives for their sensitivity in the detection of anti-neuronal nuclear antibody(Hu). Serum used for this study was taken from a patient with anti-Hu antibody seropositivity, which was ascertained by recombinant Hu protein. Western blot analysis produced 37kDa band. We examined six fixative conditions: immersion fixed with acetone, Bouin's solution, Sakura rapid fixative("Ufix'), and perfusion fixed with 2%, 4%, 8% paraformaldehyde (PFA) on the basis of each immunoreactivity in a rat cerebellum, brain stem and liver. The optimal fixation for detecting anti-Hu antibody was perfusion fixed with 2% PFA, that reacted conspicuously with nucleus but not nucleolus of neurons. The perfusion method proved superior to immersion in immunostaining intensity. With immersion fixation, specific immunostaining pattern lessened and differentiation from cytoplasm decreased. With various concentrations of PFA, immuno-reactivity with nucleus at 2% PFA was similar to 4%, although serum optimal dilution at 2% was slightly greater than 4%. The variety of staining patterns of anti-Hu antibody is closely related to the vulnerability of neuronal antigens to the fixatives. The detection of anti-neuronal antibodies is important for early diagnosis and treatment of occult tumors. The immunostaining method is a useful and sensitive way to research these antibodies. Therefore, it is essential to consider the selection of fixation and the preservation of the antigenicity in evaluating immunohistochemical hallmarks.

Animals↗
PubMed2002

[Pierre-Marie-Bamberger syndrome - a paraneoplastic syndrome of lung cancer - a case report].

The Pierre-Marie-Bamberger syndrome is a rare paraneoplastic syndrome caused by bronchial carcinoma. Typical signs are symmetric periostoses on the diaphyses of the long tubular bones, clubbed fingers and toes with eye-glass shape of the nails, neuro-vegetative disturbances and dysproteinemia. We report a 37-year-old patient with long-term nicotine abusus, who attracted attention by symptoms of a Pierre-Marie-Bamberger syndrome. Further diagnostics revealed a tumor in the apex of the left lung. After lobectomy of the upper lobe of the left lung the symptoms are completly disappeared.

Adenocarcinoma↗
PubMed1998

Paraneoplastic syndromes.

Paraneoplastic neurological syndromes cause severe neurological dysfunction and may lead to the diagnosis of a potentially curable cancer. The range of clinical presentations of these syndromes continues to grow and new antibody associations are constantly being described. Conventional immunosuppressive therapy is rarely helpful and the prognosis of these conditions remains poor.

Antigens, Neoplasm↗
PubMed2001

Two paraneoplastic syndromes in a patient with ovarian cancer: nephrotic syndrome and paraneoplastic cerebellar degeneration.

BACKGROUND: A paraneoplastic syndrome-a rare, indirect system disturbance located distant from a neoplasm-has been reported in association with ovarian cancer. CASE: Two paraneoplastic syndromes-nephrotic syndrome and paraneoplastic cerebellar degeneration (PCD)-developed in a patient who had no symptoms from ovarian cancer. She presented with the nephrotic syndrome. Evaluation for metastasis, including computed tomography (CT) scan and ultrasonography, showed a slightly enlarged right ovary and a 2-cm lower paraaortic lymph node on the right side. A 5-cm ovarian mass and multiple paraaortic lymph nodes were excised, and six cycles of carboplatin and paclitaxel were given. Nephrotic syndrome symptoms had greatly subsided at 6 weeks after surgery. At 7 months after surgery, symptoms of PCD developed yet CT, CA-125 levels, and physical examination showed no evidence of ovarian cancer. CONCLUSION: To our knowledge, development of two paraneoplastic syndromes in one patient with ovarian cancer has not been reported. Nephrotic syndrome resolved with treatment of the neoplasm whereas PCD did not.

Aged↗
PubMed1997

Mucocutaneous paraneoplastic syndromes.

Mucocutaneous paraneoplastic syndromes represent a group of dermatoses of variable morphology, pathology, and etiology that can occur as idiopathic conditions or in association with a visceral malignancy. These conditions can be categorized with respect to their predominant pathologic change: dermal "depositions," neutrophilic dermatoses, papulosquamous disorders, proliferative reactions, reactive erythemas, vacuolar degeneration of the basal layer, vasculitis, and vesiculobullous disorders. Some of these dermatoses occur more frequently in patients with hematologic malignancies whereas others are more prevalent in patients with solid tumors. The major clinical characteristics and commonly associated malignancies in patients with mucocutaneous paraneoplastic syndromes are reviewed. Suggested workups to evaluate for cancer in patients with these dermatoses are summarized. The appearance of a mucocutaneous paraneoplastic syndrome can either precede, occur concurrently with, or follow the detection of an associated neoplastic process. Therefore, the dermatosis can be the presenting feature of a previously unsuspected neoplasm or the earliest sign of recurrent cancer in an oncology patient. When the possibility of a mucocutaneous paraneoplastic syndrome is entertained, the diagnosis of the dermatosis should be confirmed either based on the clinical morphology of the lesions, the pathologic changes observed after lesional biopsy, or both. Once the diagnosis of a malignancy-associated dermatosis has been established, either an appropriate evaluation for an asymptomatic neoplasm in a cancer-free individual or an investigation for recurrence of malignancy in an oncology patient can be initiated.

Hematologic Neoplasms↗
PubMed1997

[A rheumatologic syndrome (paraneoplastic?) in benign tumors].

The authors discuss the possible development of rheumatoid paraneoplastic syndrome in patients with benign tumours. They present three observations they made (cardiac myxoma, subpleural lipoma and pulmonary fibrosclerotic pseudotumour). Paraneoplasias in these cases are interesting from the pathogenetic aspect as regards their development, possibly malignant transformation of benign intrathoracic tumours.

Adult↗
PubMed1996

Paraneoplastic syndromes.

The paraneoplastic syndromes are effects of cancer that occur at sites remote from the primary tumor and its metastases. Recognition of these disorders is important from both diagnostic and therapeutic viewpoints. The important paraneoplastic syndromes involving the endocrine, nervous, hematologic, and dermatologic systems are discussed in this article.

Humans↗
PubMed1994

Nephrotic syndrome as an unusual paraneoplastic syndrome of Hodgkin's disease demonstrated on gallium-67 scan.

Nephrotic syndrome, although rare, is recognized as one of the paraneoplastic syndromes. A patient with documented Hodgkin's disease showed increased uptake of Ga-67 in the cortex of both kidneys in addition to multiple sites of lymphomatous involvement. The patient was not receiving chemotherapy or any nephrotoxic drugs, and no other reasons that may account for renal uptake of gallium could be identified. The final clinical diagnosis was nephrotic syndrome as an unusual paraneoplastic syndrome of Hodgkin's disease. Gallium imaging was able to detect multiple sites of lymphoma and the renal uptake due to the associated nephrotic syndrome.

Adolescent↗
PubMed1994

Paraneoplastic syndromes.

The paraneoplastic syndrome does exist and its manifestations are as old as malignancy itself. Progress in molecular pathology will help us to understand its origin. At the same time, each investigator is obliged to develop therapeutic means that can be offered to the cancer patient to overcome its painful and troublesome disorders.

Adult↗